Evolution of Electrocardiographic and Structural Features Over 3 Decades in Arrhythmogenic Cardiomyopathy

A 29-year-old male was referred in 1985 for further arrhythmia management after repeated cardioversion for broad complex tachycardia that was unresponsive to numerous antiarrhythmic agents. The cause of his arrhythmia was considered a complication of viral myocarditis. His baseline ECG and echocardiogram were normal. Two different clinical tachycardias were documented during his admission, both with left bundle morphology and inferior axis, but varying cycle length (370 ms and 240 ms) and patterns of transition in the precordial leads. Invasive electrophysiological assessment demonstrated ventricular tachycardia (VT) originating in the right ventricular outflow tract and because of the risks of direct current ablation, surgical intervention was advised. At surgery, on visual inspection the right ventricular outflow tract was yellowish-grey in color and poorly contractile, and a 7×4cm section was removed, histological examination of which showed increased fibrosis. He remained well for many years controlled on dofetilide, but in 2009 he experienced a further episode of VT (Figure 1A) and after cardioversion his 12-lead ECG showed T-wave inversion to V5 with epsilon waves in leads V1 through V3. On cardiac MRI the right ventricle was dilated (115 mls/m2) with reduced function (ejection fraction 34%) but no aneurysms or free wall dyskinesia. On review of previous ECGs (Figure 2) there were no abnormalities of either …